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4. Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis

Author

Dobrota, Rucsandra, Maurer, Britta, Graf, Nicole, Jordan, Suzana, Mihai, Carina, Kowal-Bielecka, Otylia, Allanore, Yannick, Distler, Oliver, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriela, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, André, Vlachoyiannopoulos, P., Montecucco, C., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Czirják, László, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Airò, Paolo, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andréasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, F., Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Mauriziano, Ospedale, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J., Silva, José Antonio Pereira Da, Stamenkovic, Bojana, Stankovic, Aleksandra, Banja, Niska, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Müller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Clinica, U.O. Immunologia, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Walter A. Sifuentes, Midtvedt, Øyvind, Reiseter, Silje, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco P., Corrado, Ada, Pozzi, Maria R., Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Krummel-Lorenz, Brigitte, Aringer, Martin, Günther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C., de Souza Müller, Carolina, Azevedo, Valderílio F., Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O’Donnell, John, Solanki, Kamal, Veale, Douglas, OʼRourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, François, Ribi, Camillo, Buss, Guillaume, Pasquali, Jean Louis, Martin, Thierry, and Gorse, Audrey

Published
2016
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5. Rare forms of inflammatory myopathies - part II, localized forms.

Author

Galluzzo, Claudio, Chiapparoli, Ilaria, Corrado, Ada, Cantatore, Francesco Paolo, Salvarani, Carlo, and Pipitone, Nicolò

Subjects
POLYMYOSITIS, MYOSITIS, INCLUSION body myositis, NEUROMUSCULAR diseases, MUSCLE diseases, SYMPTOMS, DERMATOMYOSITIS
Abstract

The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, the anti-synthetase syndromes, immune-mediated necrotizing myopathy and inclusion body myositis. However, there are uncommon localized forms that are less known. In this review, we aimed to cover these uncommon forms. We identified rare forms of localized myositis on the basis of list provided by the homepage of the Neuromuscular disease center of Washington University, USA and on the basis of the authors' knowledge. We searched PubMed® for relevant articles on these forms with the aim of providing as much as possible information on their clinical manifestations as well as guidance on their work-up and treatment. herein, we provide un updated description of rare forms of localized myositis. These forms are often difficult to diagnose because of their localized nature and are sometimes misdiagnosed as tumors. Knowledge about these rare forms of localized myositis can aid in their recognition and treatment. [ABSTRACT FROM AUTHOR]

Published
2023
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6. Rare forms of inflammatory myopathies – part I, generalized forms.

Author

Galluzzo, Claudio, Chiapparoli, Ilaria, Corrado, Ada, Cantatore, Francesco Paolo, Salvarani, Carlo, and Pipitone, Nicolò

Subjects
POLYMYOSITIS, MYOSITIS, INCLUSION body myositis, NEUROMUSCULAR diseases, MUSCLE diseases, IMMUNE checkpoint inhibitors, SYMPTOMS
Abstract

The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, and inclusion body myositis. In this review, we aimed to cover the less common forms of generalized myositis. We identified rare forms of widespread myositis on the basis of list provided by the homepage of the Neuromuscular disease center of Washington University, USA and on the basis of the authors' knowledge. We searched PubMed® and EMBASE® for relevant articles on these forms with the aim of providing as much as possible information on their clinical manifestations as well as guidance on their work-up and treatment. There is substantial heterogeneity among the various rare forms of generalized myositis in terms of their frequency and characterization. Some forms are reasonably well defined, while others may not represent truly well-defined diseases, but rather variants of other myopathies. The landscape of rare forms appears to have evolved over time, with some forms now being better characterized, while others, such as SARS-Cov-2- and immune checkpoint inhibitor-related myositis have come to the fore only in recent years. Knowledge about rare forms of myositis can aid in their recognition and treatment. [ABSTRACT FROM AUTHOR]

Published
2023
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7. Digital ulcers predict a worse disease course in patients with systemic sclerosis

Author

Mihai, Carina, Landewé, Robert, van der Heijde, Désirée, Walker, Ulrich A, Constantin, Paul I, Gherghe, Ana Maria, Ionescu, Ruxandra, Rednic, Simona, Allanore, Yannick, Avouac, Jérôme, Czirják, László, Hachulla, Eric, Riemekasten, Gabriela, Cozzi, Franco, Airò, Paolo, Cutolo, Maurizio, Mueller-Ladner, Ulf, Matucci-Cerinic, Marco, Launay, David, Dobrotă, Rucsandra, Sfrenţ-Cornăţeanu, Roxana, Zingarelli, Stefania, Pigatto, Erika, Cuomo, Giovanna, Caramaschi, Paola, Ananieva, Lidia, Ullman, Susanne, Iversen, Line, Gurman, Alexandra Balbir, Braun-Moscovici, Yolanda, Carreira, Patricia E., Joven, Beatriz E, Minier, Tünde, Guiducci, Serena, Bellando-Randone, Silvia, Pellerito, Raffaele, Hunzelmann, Nicolas, Tarner, Ingo H., Radominski, Sebastião Cezar, de Souza Müller, Carolina, Iannone, Florenzo, Henes, Jörg, Bancel, Dominique Farge, Damjanov, Nemanja, Ostojić, Predrag, Pozzi, Maria Rosa, Hesselstrand, Roger, Denton, Christopher, Krasowska, Dorota, Tikly, Mohammed, Riccieri, Valeria, Cantatore, Francesco Paolo, Corrado, Ada, Da Silva, José Antonio Pereira, Salvador, Maria João, Tyndall, Alan, Gabrielli, Armando, Distler, Oliver, Jordan, Suzan, Heitmann, Stefan, Burkhardt, Harald, Himsel, Andrea, Rozman, Blaz, Smith, Vanessa, De Keyser, Filip, Kalitena, Dusanka Martinovic, Radic, Mislav, Filipescu, Ileana, Petcu, Ana, Vlachoyiannopoulos, Panayiotis, Kucharz, Eugene J., Widuchowska, Malgorzata, Kopec-Medrek, Magdalena, Kotulska, Anna, Szücs, Gabriella, Stankovic, Aleksandra, Stamenkovic, Bojana, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Coleiro, Bernard, Santamaria, Vera Ortiz, Westhovens, René, Bečvář, Radim, Novak, Srdan, Engelhart, Merete, Meroni, Pierluigi, Ingegnoli, Francesca, Zeni, Silvana, Sulli, Alberto, Distler, Jörg, Yavuz, Sule, Montecucco, Carlomaurizio, Eyerich, Kilian, Krummel-Lorenz, Brigitte, Zenone, Thierry, Midtvedt, Øyvind, Chizzolini, Carlo, Seidel, Matthias, Oleszowsky, Mara, Üprus, Maria, Opriş, Daniela, Groşeanu, Laura, Bielecka, Otylia Kowal, Antonio, Zea Mendoza, Szechinski, Jacek, Morović-Vergles, Jadranka, Scorza, Raffaella, Puppo, Francesco, Mathieu, Alessandro, Anic, Branimir, Stork, Jiri, Stebbings, Simon, Inanc, Murat, Hasler, Paul, von Mühlen, Carlos Alberto, Aringer, Martin, Popa, Sergei, Li, Mengtao, and Rosato, Edoardo

Published
2016
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8. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

Author

Elhai, Muriel, Avouac, Jérôme, Walker, Ulrich A, Matucci-Cerinic, Marco, Riemekasten, Gabriela, Airò, Paolo, Hachulla, Eric, Valentini, Gabriele, Carreira, Patricia E, Cozzi, Franco, Balbir Gurman, Alexandra, Braun-Moscovici, Yolanda, Damjanov, Nemanja, Ananieva, Lidia P, Scorza, Raffaella, Jimenez, Sergio, Busquets, Joanna, Li, Mengtao, Müller-Ladner, Ulf, Kahan, André, Distler, Oliver, Allanore, Yannick, Guiducci, Serena, Tyndall, Alan, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Cuomo, Giovanna, Vettori, Serena, Rednic, Simona, Nicoara, Ileana, Vlachoyiannopoulos, P., Montecucco, C., Caporali, Roberto, Novak, Srdan, Czirják, László, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Govoni, M., Monaco, Andrea Lo, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Denton, Christopher, Henes, Jörg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N., Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Zea Mendoza, Antonio, de la Puente Buijdos, Carlos, Sifuentes Giraldo, Walter A., Midtvedt, Øyvind, Garen, Torhild, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M., Mihai, Carmen M., Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Smith, Vanessa, Cantatore, Francesco P., Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R., Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C., Müller, Carolina de Souza, Azevedo, Valderílio F., Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Stebbings, Simon, Highton, John, Stamp, Lisa, Chapman, Peter, Baron, Murray, OʼDonnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, OʼRourke, Marie, Loyo, Esthela, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E., Kafaja, Suzanne, Lefebvre, Paloma García de la Peña, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Kerzberg, Eduardo, Montoya, Fabiana, and Cosentino, Vanesa

Published
2016
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9. Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study

Author

Avouac, Jérôme, Walker, Ulrich A, Hachulla, Eric, Riemekasten, Gabriela, Cuomo, Giovanna, Carreira, Patricia E, Caramaschi, Paola, Ananieva, Lidia P, Matucci-Cerinic, Marco, Czirjak, Laszlo, Denton, Christopher, Ladner, Ulf Müller, Allanore, Yannick, Guiducci, Serena, Tyndall, Alan, Lapadula, Giovanni, Iannone, Florenzo, Distler, Oliver, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Vlachoyiannopoulos, Panayiotis G., Montecucco, Carlomaurizio, Caporali, Roberto, Novak, Srdan, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge-Bancel, Dominique, Hadj-Khelifa, Sondess, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Martinovic, Duska, Gurman, Alexandra Balbir, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Kötter, Ina, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Silva, José Antonio Pereira Da, Salvador, Maria João, Stamenkovi, Bojana, Stankovic, Aleksandra, Marasini, Bianca, Belloli, Laura, Tikly, Mohammed, Denisov, Lev N., Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Szücs, Gabriella, Szamosi, Szilvia, de la Puente, Carlos, de la Pena Lefebvre, Paloma García, Midtvedt, Øyvind, Garen, Torhild, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Wigley, Fredrick M., Stoica, Viktor, Mihai, Carmen Marina, Distler, Jörg, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Rüdiger, Szechinski, Jacek, Wiland, Piotr, Krummel-Lorenz, Brigitte, Aringer, Martin, Westhovens, Rene, De Langhe, Ellen, Anic, Branimir, Baresi, Marko, Üprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastião Cezar, de Souza Müller, Carolina, Jimenez, Sergio, Busquets, Joanna, Popa, Sergei, Agachi, Svetlana, Zenone, Thierry, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Foti, Rosario, and Rubio, Silvia Rodriguez

Published
2016
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11. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

Author

Maurer, Britta, Graf, Nicole, Michel, Beat A, Müller-Ladner, Ulf, Czirják, László, Denton, Christopher P, Tyndall, Alan, Metzig, Carola, Lanius, Vivian, Khanna, Dinesh, Distler, Oliver, Tarner, Ingo H, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriele, Rednic, Simona, Nicoara, Ileana, Kahan, André, Allanore, Yannick, Vlachoyiannopoulos, P, Montecucco, C, Caporali, Roberto, Carreira, Patricia E, Novak, Srdan, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J, Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Gurman, Alexandra Balbir, Braun-Moscovici, Yolanda, Govoni, M, Monaco, Andrea Lo, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J, Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N, Ananieva, Lidia P, Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Mendoza, Antonio Zea, Buijdos, Carlos de la Puente, Sifuentes Giraldo, Walter A., Midtvedt, Øyvind, Garen, Torhild, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Mihai, Carmen M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Cantatore, Francesco P, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R, Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C, Müller, Carolina de Souza, Azevedo, Valderílio F, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, OʼDonnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O’Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Chirieac, Rodica, Ancuta, Codrina, E Furst, Daniel, Kafaja, Suzanne, Lefebvre, Paloma García de la Peña, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Kerzberg, Eduardo, Montoya, Fabiana, and Cosentino, Vanesa

Published
2015
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13. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the european scleroderma trials and research (eustar) cohort

Author

Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, W., Jordan, S., Graf, N., de Oliveira Pena, J., Curram, J., Allanore, Y., Matucci-Cerinic, M., Pope, J. E., Denton, C. P., Khanna, D., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., Sarzi Puttini, P., Ege Üniversitesi, Chizzolini, Carlo, Allali, Danièle, University of Zurich, and Distler, Oliver

Subjects
INVOLVEMENT, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, Time Factors, Databases, Factual, Skin Diseases/etiology/mortality/physiopathology, PREDICTION, Fibrosi, 2745 Rheumatology, Diffuse/complications/mortality/pathology, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, Kaplan-Meier Estimate, ddc:616.07, Severity of Illness Index, Scleroderma, Cohort Studies, PROGNOSTIC-FACTORS, Fibrosis, Medicine and Health Sciences, scleroderma, Lung, Skin, integumentary system, progressive skin fibrosis, Lung function decline, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, DEATH, Middle Aged, ddc, Europe, VARIABILITY, factual, Cohort, Visceral organ progression, 2723 Immunology and Allergy, Disease Progression, Female, Survival Analysi, Life Sciences & Biomedicine, Cohort study, Human, Adult, Skin/pathology, medicine.medical_specialty, databases, All-cause death, risk analysis, diffuse cutaneous systemic sclerosis, 610 Medicine & health, IMPROVEMENT, Systemic Sclerosis, Skin Diseases, THICKNESS SCORE, VALIDATION, Databases, FEV1/FVC ratio, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, Humans, Factual, Survival analysis, 2403 Immunology, Science & Technology, Proportional hazards model, business.industry, Surrogate endpoint, MORTALITY, Skin Disease, fibrosis, Progressive skin fibrosi, Lung/physiopathology, biomarkers, Diffuse cutaneous systemic sclerosi, medicine.disease, Survival Analysis, all-cause death, lung function decline, visceral organ progression, adult, cohort studies, databases, factual, disease progression, female, humans, Scleroderma, Diffuse, Cohort Studie, business
Abstract

PubMed: 30852552, Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ?7, valid mRSS at 12±3 months after baseline and ?1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ?25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ?10% (53.6% vs 34.4%; p, Bayer Bayer, 1Department of Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Clinical Development Pulmonology, Bayer Us llC, Whippany, new Jersey, Usa 4Data science and analytics, Bayer plc, Reading, UK 5Rheumatology a Department, Paris Descartes University, inseRM U1016, sorbonne, Paris Cité, Cochin Hospital, Paris, France 6Division of Rheumatology, University of Florence, Florence, italy 7Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Western Ontario, Canada 8Department of Rheumatology, Royal Free Hospital, University College london, london, UK 9scleroderma Program, Department of internal Medicine, Division of Rheumatology, University of Michigan, ann arbor, Michigan, Usa Acknowledgements The authors thank nicole schneider for excellent administration and data entry into the eUsTaR cohort. Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., This study was supported by a grant from Bayer aG.

Published
2019

14. Phenotypes determined by cluster analysis and their survival in the prospective European Scleroderma Trials and Research cohort of patients with systemic sclerosis

Author

Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E, Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J, Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P, Denisov, Lev N, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D, Yoshinari, Natalino H, Marangoni, Roberta G, Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C, Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A, Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, Poindron, Vincent, EUSTAR Collaborators, Guiducci, S., Walker, U., Kyburz, D., Lapadula, G., Iannone, F., Maurer, B., Jordan, S., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Siegert, E., Rednic, S., Nicoara, I., Kahan, A., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Stork, J., Inanc, M., Carreira, P.E., Novak, S., Czirják, L., Varju, C., Chizzolini, C., Kucharz, E.J., Kotulska, A., Kopec-Medrek, M., Widuchowska, M., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Wu, C., Marjanovic, Z., Faivre, H., Hij, D., Dhamadi, R., Airò, P., Hesselstrand, R., Wollheim, F., Wuttge, D.M., Andréasson, K., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Trotta, F., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Mauriziano, O., Maria Bambara, L., Caramaschi, P., Morovic-Vergles, J., Black, C., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Burkhardt, H., Himsel, A., Bajocchi, G., Maria Nuova, A.S., João Salvador, M., Pereira Da Silva, J.A., Stamenkovic, B., Stankovic, A., Francesco Selmi, C., De Santis, M., Marasini, B., Tikly, M., Ananieva, L.P., Denisov, L.N., Müller-Ladner, U., Frerix, M., Tarner, I., Scorza, R., Puppo, F., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szücs, G., Szamosi, S., Zea Mendoza, A., de la Puente, C., Sifuentes Giraldo, W.A., Midtvedt, Ø., Reiseter, S., Garen, T., Valesini, G., Riccieri, V., Maria Ionescu, R., Opris, D., Groseanu, L., Wigley, F.M., Sfrent Cornateanu, R., Ionitescu, R., Maria Gherghe, A., Soare, A., Gorga, M., Bojinca, M., Mihai, C., Milicescu, M., Sunderkötter, C., Kuhn, A., Sandorfi, N., Schett, G., Distler, J.H., Beyer, C., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Paolo Cantatore, F., Corrado, A., Ullman, S., Iversen, L., Alberto von Mühlen, C., Marilu Bohn, J., Scussel Lonzetti, L., Rosa Pozzi, M., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Houssiau, F.A., Jose Alegre-Sancho, J., Krummel-Lorenz, B., Saar, P., Aringer, M., Günther, C., Westhovens, R., de Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Üprus, M., Otsa, K., Yavuz, S., Granel, B., Cezar Radominski, S., de Souza Müller, C., Azevedo, V.F., Jimenez, S., Busquets, J., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Popa, S., Zenone, T., Pileckyte, M., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Sampaio-Barros, P.D., Yoshinari, N.H., Marangoni, R.G., Martin, P., Fuocco, L., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Abdel Atty Mohamed, W.A., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C.M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Chirieac, R., Ancuta, C., Furst, D.E., Villiger, P., Adler, S., van Laar, J., Kayser, C., Eduardo C, A.L., Fathi, N., Hassanien, M., de la Peña Lefebvre, P.G., Rodriguez Rubio, S., Valero Exposito, M., Sibilia, J., Chatelus, E., Gottenberg, J.E., Chifflot, H., Litinsky, I., Emery, P., Buch, M., Del Galdo, F., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Ann Saketkoo, L., Lasky, J.A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Luca Brucato, A., Lupi, E., Rosner, I., Rozenbaum, M., Slobodin, G., Boulman, N., Rimar, D., Couto, M., Spertini, F., Ribi, C., Buss, G., Kahl, S., Hsu, V.M., Chen, F., McCloskey, D., Malveaux, H., Louis Pasquali, J., Martin, T., Gorse, A., Guffroy, A., Poindron, V., and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, Databases, Factual, systemic sclerosis, SUBSETS, Disease, Severity of Illness Index, Scleroderma, DISEASE, 0302 clinical medicine, Medicine and Health Sciences, Immunology and Allergy, Cluster Analysis, CRITERIA, Prospective Studies, Prospective cohort study, skin and connective tissue diseases, integumentary system, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, Adult, Aged, Autoantibodies/blood, Europe/epidemiology, Female, Humans, Middle Aged, Phenotype, Prognosis, Scleroderma, Diffuse/blood, Scleroderma, Diffuse/epidemiology, Scleroderma, Diffuse/pathology, Scleroderma, Limited/blood, Scleroderma, Limited/epidemiology, Scleroderma, Limited/pathology, Scleroderma, Systemic/blood, Scleroderma, Systemic/epidemiology, Scleroderma, Systemic/pathology, Connective tissue disease, ddc, Europe, MANIFESTATIONS, Cohort, Life Sciences & Biomedicine, medicine.medical_specialty, Immunology, PROFILE, CLASSIFICATION, 03 medical and health sciences, Rheumatology, Scleroderma, Limited, Internal medicine, Severity of illness, medicine, Autoantibodies, 030203 arthritis & rheumatology, Science & Technology, Scleroderma, Systemic, business.industry, Autoantibody, Systemic sclerosis (SSc), medicine.disease, 030104 developmental biology, Scleroderma, Diffuse, business
Abstract

OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSION: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Published
2019

15. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: A European Scleroderma Trials and Research (EUSTAR) analysis

Author

Becker, Mike, Graf, Nicole, Sauter, Rafael, Allanore, Yannick, Curram, John, Denton, Christopher P., Khanna, Dinesh, Matucci-Cerinic, Marco, Pena, Janethe de Oliveira, Pope, Janet E., Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Ege Üniversitesi, University of Zurich, Distler, Oliver, Chizzolini, Carlo, Allai, Daniela, Becker, M., Graf, N., Sauter, R., Allanore, Y., Curram, J., Denton, C. P., Khanna, D., Matucci-Cerinic, M., de Oliveira Pena, J., Pope, J. E., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., and Sarzi Puttini, P.

Subjects
INVOLVEMENT, SELECTION, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, predictive factors, systemic sclerosis, 2745 Rheumatology, epidemiologic methods, morbidity, Disease, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, INTERSTITIAL LUNG-DISEASE, disease worsening, mortality, predictive factors, systemic sclerosis, predictive factor, disease worsening, DESIGN, middle aged, Medicine and Health Sciences, FIBROSIS, scleroderma, SKIN THICKNESS SCORE, mortality, skin and connective tissue diseases, Prospective cohort study, humans, lung diseases, ddc:616, education.field_of_study, heart diseases, integumentary system, clinical trials as topic, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, Interstitial lung disease, follow-up studies, ddc, female, Cohort, 2723 Immunology and Allergy, europe, Life Sciences & Biomedicine, CLINICAL-TRIALS, survival rate, medicine.medical_specialty, Population, 610 Medicine & health, disease progression, male, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, REGRESSION, medicine, severity of illness index, education, Survival rate, METAANALYSIS, 2403 Immunology, Science & Technology, Scleroderma, Systemic, business.industry, MORTALITY, systemic, medicine.disease, prospective studies, Clinical trial, prognosis, scleroderma, diffuse, scleroderma, systemic, Scleroderma, Diffuse, business
Abstract

PubMed: 31227488, Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12±3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ., Bayer Bayer, United Kingdom Université Paris Descartes Li Ka Shing Foundation, LKSF University of Michigan, U-M, 1Department of Rheumatology and the Centre of experimental Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Big Data institute, li Ka shing Centre for Health information and Discovery, nuffield Department of Medicine, University of Oxford, Oxford, UK 4Rheumatology a Department, Paris Descartes University, sorbonne Paris Cité, Cochin Hospital, Paris, France 5Data science and analytics, Bayer plc, Reading, UK 6UCl Division of Medicine, Royal Free Campus, london, UK 7Division of Rheumatology, Department of internal Medicine, University of Michigan scleroderma Program, University of Michigan, ann arbor, Michigan, Usa 8Department of experimental and Clinical Medicine, University of Florence, Florence, italy 9Bayer Us llC, Whippany, new Jersey, Usa 10Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Ontario, Canada Acknowledgements The R-code for the linear Mi-lassO was received from Qixuan Chen.21 Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., Contributors study conception and design, acquisition of data, analysis and interpretation of data and drafting and revising the article: OD and MB; analysis and interpretation of data: OD, MB, Rs and nG. all authors have critically reviewed and approved the final submitted version to be published. Funding This study was supported by a grant from Bayer aG. Bayer employees are coauthors of this paper and supported the study design and interpretation of the data, but otherwise Bayer had no influence on the study., Competing interests MOB declares no conflict of interest. OD has had consultancy relationships with actelion, Bayer, Biogen idec, Boehringer ingelheim, Chemomab, espeRare foundation, Genentech/Roche, GsK, inventiva, italfarmaco, lilly, medac, Medimmune, Mitsubishi Tanabe Pharma, Pharmacyclics, novartis, Pfizer, sanofi, sinoxa and UCB in the area of potential treatments of scleroderma and its complications. OD has received research funding from actelion, Bayer, Boehringer ingelheim, Mitsubishi Tanabe Pharma and Roche in the area of potential treatments of scleroderma and its complications. OD has a patent for mir-29 licensed for the treatment of systemic sclerosis. DK has consultancy relationships and/or has received grant/research support from Bayer, Bristol-Myers squibb, Boehringer ingelheim, Genentech/Roche, niH, Pfizer, sanofi-aventis Pharmaceuticals, actelion Pharmaceuticals Us, Chemomab, Corbus, Covis, Cytori, eicos, eMD serono, Gilead, GlaxosmithKline, and UCB Pharma. He is a shareholder of eicos. CPD has consultancy relationships with and/or has received speakers’ bureau fees from actelion Pharmaceuticals Us, Bayer aG, GlaxosmithKline, Csl Behring, Merck serono, Roche Pharmaceuticals, Genentech and Biogen iDeC inc., inventiva, sanofi-aventis Pharmaceuticals and Boehringer ingelheim. JeP has consultancy relationships with and/or has received grant/research support from actelion, Bayer aG, Bristol-Myers squibb, Merck, Pfizer inc. and Roche. MM-C has consultancy relationships and/ or has received grant/research support from Pfizer, Bristol-Myers squibb, actelion, UCB Pharma, Bayer, Chemomab, Genentech/Roche, inventiva and lilly. Ya has consultancy relationships with and/or has received grant/research support from actelion, Pharmaceuticals Us, Bayer aG, Bristol-Myers squibb, inventiva, Medac, Pfizer inc., Roche Pharmaceuticals, Genentech and Biogen iDeC inc., sanofi-aventis Pharmaceuticals and servier. JdOP and JC are employees of Bayer. nTG has nothing to disclose.

Published
2019

16. Digital ulcers predict a worse disease course in patients with systemic sclerosis

Author

Mihai, Carina, Landewé, Robert, Van Der Heijde, Désirée, Walker, Ulrich A., Constantin, Paul I., Gherghe, Ana Maria, Ionescu, Ruxandra, Rednic, Simona, Allanore, Yannick, Avouac, Jéroˆme, Czirják, László, Hachulla, Eric, Riemekasten, Gabriela, Cozzi, Franco, Airò, Paolo, Cutolo, Maurizio, Mueller Ladner, Ulf, Matucci Cerinic, Marco, Launay, David, Dobrota, Rucsandra, Sfrent Cornateanu, Roxana, Zingarelli, Stefania, Pigatto, Erika, Cuomo, Giovanna, Caramaschi, Paola, Ananieva, Lidia, Ullman, Susanne, Iversen, Line, Gurman, Alexandra Balbir, Braun Moscovici, Yolanda, Carreira, Patricia E., Joven, Beatriz E., Minier, Tünde, Guiducci, Serena, Bellando Randone, Silvia, Pellerito, Raffaele, Hunzelmann, Nicolas, Tarner, Ingo H., Radominski, Sebastião Cezar, De Souza Müller, Carolina, Iannone, Florenzo, Henes, Jörg, Bancel, Dominique Farge, Damjanov, Nemanja, Ostojic, Predrag, Pozzi, Maria Rosa, Hesselstrand, Roger, Denton, Christopher, Krasowska, Dorota, Tikly, Mohammed, Riccieri, Valeria, Cantatore, Francesco Paolo, Corrado, Ada, Da Silva, José Antonio Pereira, Salvador, Maria João, Tyndall, Alan, Gabrielli, Armando, Distler, Oliver, Jordan, Suzan, Heitmann, Stefan, Burkhardt, Harald, Himsel, Andrea, Rozman, Blaz, Smith, Vanessa, Keyser, Filip De, Kalitena, Dusanka Martinovic, Radic, Mislav, Filipescu, Ileana, Petcu, Ana, Vlachoyiannopoulos, Panayiotis, Kucharz, Eugene J., Widuchowska, Malgorzata, Kopec Medrek, Magdalena, Kotulska, Anna, Szücs, Gabriella, Stankovic, Aleksandra, Stamenkovic, Bojana, Selmi, Carlo Francesco, Santis, Maria De, Marasini, Bianca, Coleiro, Bernard, Santamaria, Vera Ortiz, Westhovens, René, Becvár, Radim, Novak, Srdan, Engelhart, Merete, Meroni, Pierluigi, Ingegnoli, Francesca, Zeni, Silvana, Sulli, Alberto, Distler, Jörg, Yavuz, Sule, Montecucco, Carlomaurizio, Eyerich, Kilian, Krummel Lorenz, Brigitte, Zenone, Thierry, Midtvedt, Øyvind, Chizzolini, Carlo, Seidel, Matthias, Oleszowsky, Mara, Üprus, Maria, Opriş, Daniela, Groseanu, Laura, Bielecka, Otylia Kowal, Antonio, Zea Mendoza, Szechinski, Jacek, Morovic Vergles, Jadranka, Scorza, Raffaella, Puppo, Francesco, Mathieu, Alessandro, Anic, Branimir, Stork, Jiri, Stebbings, Simon, Inanc, Murat, Hasler, Paul, Von Mühlen, Carlos Alberto, Aringer, Martin, Popa, Sergei, Mengtao, Li, Rosato, Edoardo, University of Zurich, and Mihai, Carina

Subjects
Genetics and Molecular Biology (all), Lung Diseases, Male, 0301 basic medicine, Databases, Factual, Epidemiology, 2745 Rheumatology, Vital Capacity, Disease, Biochemistry, Scleroderma, Systemic autoimmune disease, High morbidity, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Medicine (all), 10051 Rheumatology Clinic and Institute of Physical Medicine, Systemic Sclerosis, Adult, Aged, Cardiovascular Diseases, Disease Progression, Female, Hand Dermatoses, Humans, Hypertension, Pulmonary, Kidney Diseases, Middle Aged, Multivariate Analysis, Prognosis, Proportional Hazards Models, Pulmonary Diffusing Capacity, Retrospective Studies, Scleroderma, Systemic, Skin Ulcer, Stroke Volume, Fingers, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Orvostudományok, Pulmonary, Hypertension, 2723 Immunology and Allergy, medicine.medical_specialty, 610 Medicine & health, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, Disease course, Databases, 03 medical and health sciences, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Cox proportional hazards regression, medicine, In patient, Factual, 030203 arthritis & rheumatology, 2403 Immunology, business.industry, Systemic, medicine.disease, 030104 developmental biology, Physical therapy, business
Abstract

ObjectiveSystemic sclerosis (SSc) is a systemic autoimmune disease with high morbidity and significant mortality. There is a great need of predictors that would allow risk stratification of patients with SSc and ultimately initiation of treatment early enough to ensure optimal clinical results. In this study, we evaluated whether a history of digital ulcers (HDU) at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death in patients with SSc.MethodsPatients from the EULAR Scleroderma Trials and Research (EUSTAR) database, satisfying at inclusion the 1980 American College of Rheumatology classification criteria for SSc, who had a follow-up of at least 3 years since baseline or who have died, were included in the analysis. HDU at presentation as a predictor of disease worsening or death was evaluated by Cox proportional hazards regression analysis.Results3196 patients matched the inclusion criteria (male sex 13.2%, 33.4% diffuse subset). At presentation, 1092/3196 patients had an HDU (34.1%). In multivariable analysis adjusting for age, gender and all parameters considered potentially significant, HDU was predictive for the presence of active digital ulcers (DUs) at prospective visits (HR (95% CI)): 2.41 (1.91 to 3.03), pConclusionsIn patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival.

Published
2015

17. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: A longitudinal EUSTAR study

Author

Jaeger, Veronika K., Wirz, Elina G., Allanore, Yannick, Rossbach, Philipp, Riemekasten, Gabriela, Hachulla, Eric, Distler, Oliver, Airò, Paolo, Carreira, Patricia E., Gurman, Alexandra Balbir, Tikly, Mohammed, Vettori, Serena, Damjanov, Nemanja, Müller Ladner, Ulf, Distler, Jörg H. W., Mangtao, Li, Walker, Ulrich A., Ananieva, Lidia, Heitmann, Stefan, Rednic, Simona, Riccieri, Valeria, Szmyrka Kaczmarek, Magdalena, Farge, Dominique, Lapadula, Giovanni, Matucci Cerinic, Marco, Guiducci, Serena, Hunzelmann, Nicolas, Ricci, Massimo, Mihai, Carina, Veale, Douglas, Hesselstrand, Roger, Mariok, Eduardo, Smith, Vanessa, Tarner, Ingo H., Kucharz, Eugene J., Czirják, László, Martinovic, Duska, Solanki, Kamal, Ancuta, Codrina Mihaela, Sibilia, Jean, Paola, Caramaschi, Hassanien, Manal, Kahl, Sarah, Wigley, Frederick, Vanthuyne, Marie, Opris, Daniela, Radominski, Sebastião C., Monaco, Andrea Lo, Corrado, Ada, Koehm, Michaela, Codullo, Veronica, Radim, Bevcar, Loyo, Esthela, Üprus, Maria, Pellerito, Raffaele, Zenone, Thierry, Gabrielli, Armando, Kowal Bielecka, Otylia, Rozman, Blaz, Scorza, Raffaella, Saketkoo, Lesley Ann, Midtvedt, Oyvind, Von Mühlen, Carlos Alberto, Henes, Jörg, Branimir, Ani, Hasler, Paul, Yavuz, Sule, Adler, Sabine, Krummel Lorenz, Brigitte, Posa, Magdalena, Engelhart, Merete, Denton, Christopher, Krasowska, Dorota, Lefebvre, Paloma Garcia De La Peña, Cozzi, Franco, Mouthon, Luc, Rosato, Edoardo, Selmi, Carlo, Sancho, Juan José Alegre, Mallia, Carmel, Limonta, Massimiliano, Seidel, Matthias, Foti, Rosario, Stamp, Lisa, Ullman, Susanne, Stebbings, Simon, Santamaria, Vera Ortiz, Del Galdo, Francesco, De Langhe, Ellen, Mathieu, Alessandro, Sunderkötter, Cord, Eyerich, Kilian, Stamenkovic, Bojana, Novak, Srdan, Sampaio Barros, Percival D., Kayser, Cristiane, Litinsky, Ira, Couto, Maura, Jaeger, Veronika K., Wirz, Elina G., Allanore, Yannick, Rossbach, Philipp, Riemekasten, Gabriela, Hachulla, Eric, Distler, Oliver, Airã², Paolo, Carreira, Patricia E., Gurman, Alexandra Balbir, Tikly, Mohammed, Vettori, Serena, Damjanov, Nemanja, Mã¼ller ladner, Ulf, Distler, Jã¶rg H. W., Li, Mangtao, Walker, Ulrich A., Ananieva, Lidia, Heitmann, Stefan, Rednic, Simona, Riccieri, Valeria, Szmyrka kaczmarek, Magdalena, Farge, Dominique, Lapadula, Giovanni, Matucci cerinic, Marco, Guiducci, Serena, Hunzelmann, Nicola, Ricci, Massimo, Mihai, Carina, Veale, Dougla, Hesselstrand, Roger, Mariok, Eduardo, Smith, Vanessa, Tarner, Ingo H., Kucharz, Eugene J., Czirjã¡k, Lã¡szlã³, Martinovic, Duska, Solanki, Kamal, Ancuta, Codrina Mihaela, Sibilia, Jean, Paola, Caramaschi, Hassanien, Manal, Kahl, Sarah, Wigley, Frederick, Vanthuyne, Marie, Opris, Daniela, Radominski, Sebastiã£o C., Monaco, Andrea Lo, Corrado, Ada, Koehm, Michaela, Codullo, Veronica, Radim, Bevcar, Loyo, Esthela, Ãœprus, Maria, Pellerito, Raffaele, Zenone, Thierry, Gabrielli, Armando, Kowal bielecka, Otylia, Rozman, Blaz, Scorza, Raffaella, Saketkoo, Lesley Ann, Midtvedt, Oyvind, Von Mã¼hlen, Carlos Alberto, Henes, Jã¶rg, Branimir, Ani, Hasler, Paul, Yavuz, Sule, Adler, Sabine, Krummel lorenz, Brigitte, Posa, Magdalena, Engelhart, Merete, Denton, Christopher, Krasowska, Dorota, Lefebvre, Paloma Garcia De La Peã±a, Cozzi, Franco, Mouthon, Luc, Rosato, Edoardo, Selmi, Carlo, Sancho, Juan Josã© Alegre, Mallia, Carmel, Limonta, Massimiliano, Seidel, Matthia, Foti, Rosario, Stamp, Lisa, Ullman, Susanne, Stebbings, Simon, Santamaria, Vera Ortiz, Del Galdo, Francesco, De Langhe, Ellen, Mathieu, Alessandro, Sunderkã¶tter, Cord, Eyerich, Kilian, Stamenkovic, Bojana, Novak, Srdan, Sampaio barros, Percival D., Kayser, Cristiane, Litinsky, Ira, Couto, Maura, and University of Zurich

Subjects
Male, Genetics and Molecular Biology (all), Pulmonology, Blood Pressure, Vascular Medicine, Biochemistry, Scleroderma, Diagnostic Radiology, 0302 clinical medicine, Retrospective Studie, Longitudinal Studies, lcsh:Science, integumentary system, Incidence, Arteries, Systolic Pressure, 3. Good health, Echocardiography, Cohort, Regression Analysis, Human, medicine.medical_specialty, Imaging Techniques, Gastrointestinal Disease, Immunology, 1100 General Agricultural and Biological Sciences, Skin Diseases, 03 medical and health sciences, FEV1/FVC ratio, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Humans, Autoantibodies, Retrospective Studies, Aged, 1000 Multidisciplinary, Biochemistry, Genetics and Molecular Biology (all), Proportional hazards model, lcsh:R, Biology and Life Sciences, Proteins, medicine.disease, Agricultural and Biological Sciences (all), Blood Vessels, lcsh:Q, Clinical Medicine, Ejection Fraction, Lung Diseases, Kidney Disease, Physiology, Gastrointestinal Diseases, lcsh:Medicine, Longitudinal Studie, Kaplan-Meier Estimate, Medicine (all), Lung Disease, Pericardial effusion, Risk Factors, Medizinische Fakultät, Immune Physiology, Ultrasound Imaging, Medicine and Health Sciences, 030212 general & internal medicine, Pulmonary Arteries, Connective Tissue Diseases, Immune System Proteins, Pulmonary Hypertension, Multidisciplinary, Ejection fraction, Radiology and Imaging, Incidence (epidemiology), 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Autoantibodie, Heart Disease, Kidney Diseases, Female, Anatomy, Research Article, Adult, Heart Diseases, Cardiology, 610 Medicine & health, Research and Analysis Methods, Antibodies, Regression Analysi, Autoimmune Diseases, Diagnostic Medicine, Internal medicine, medicine, ddc:610, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, Risk Factor, Skin Disease, Raynaud Disease, Retrospective cohort study, Surgery, Cardiovascular Anatomy, Clinical Immunology, business
Abstract

Objective Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. Methods In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. Results Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. Conclusion In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to ‘widen' the still very narrow ‘window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.

Published
2016

18. Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis

Author

Carreira, Patricia E, et al, Distler, Oliver, Farge, Dominique, Hesselstrand, Roger, Corrado, Ada, Caramaschi, Paola, Tikly, Mohammed, Matucci-Cerinic, Marco, EUSTAR co-authors, University of Zurich, and Carreira, Patricia E

Subjects
2403 Immunology, 2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, 610 Medicine & health
Published
2017

19. The incidence of cardiovascular events in Italian patients with systemic lupus erythematosus is lower than in North European and American cohorts

Author

Fasano, Serena, primary, Margiotta, Domenico Paolo, additional, Gualtierotti, Roberta, additional, Corrado, Ada, additional, Berardicurti, Onorina, additional, Iacono, Daniela, additional, Pierro, Luciana, additional, Riccardi, Antonella, additional, Giacomelli, Roberto, additional, Cantatore, Francesco Paolo, additional, Meroni, Pier Luigi, additional, Afeltra, Antonella, additional, and Valentini, Gabriele, additional

Published
2018
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20. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

Author

Ingegnoli, Francesca, primary, Boracchi, Patrizia, additional, Gualtierotti, Roberta, additional, Smith, Vanessa, additional, Cutolo, Maurizio, additional, Foeldvari, Ivan, additional, Airò, Paolo, additional, Alegre-Sancho, Juan Jose, additional, Allanore, Yannick, additional, Ananieva, Lidia P., additional, Ancuta, Codrina, additional, Andrade, Luis Eduardo, additional, Aringer, Martin, additional, Becvar, Radim, additional, Bojinca, Mihai, additional, Butrimiene, Irena, additional, Cantatore, Francesco Paolo, additional, Caporali, Roberto, additional, Caramaschi, Paola, additional, Carreira, Patricia E., additional, Chirieac, Rodica, additional, Corrado, Ada, additional, Cosentino, Vanesa, additional, Cuomo, Giovanna, additional, Czirjak, Laszlo, additional, Da Silva, José Antonio Pereira, additional, la Peña Lefebvre, Paloma García de, additional, De Keyser, Filip, additional, de Souza Müller, Carolina, additional, Damgaard, Kirsten, additional, Damjanov, Nemanja, additional, Denisov, Lev N., additional, Distler, Oliver, additional, Doube, Alan, additional, Dumitrascu, Alina, additional, Engelhart, Merete, additional, Exposito, Marta Valero, additional, Eyerich, Kilian, additional, Farge-Bancel, Dominique, additional, Azevedo, Valderílio Feijó, additional, Foti, Rosario, additional, Frerix, Marc, additional, Gabrielli, Armando, additional, Garen, Torhild, additional, Gottschalk, Paola, additional, Groseanu, Laura, additional, Guiducci, Serena, additional, Günther, Claudia, additional, Hachulla, Eric, additional, Hein, Rüdiger, additional, Heitmann, Stefan, additional, Henes, Jörg, additional, Hesselstrand, Roger, additional, Highton, John, additional, Iannone, Florenzo, additional, Ionescu, Ruxandra Maria, additional, Kayser, Cristiane, additional, Karpec, Diana, additional, Kerzberg, Eduardo, additional, Kotulska, Anna, additional, Kopec-Medrek, Magdalena, additional, Kucharz, Eugene, additional, Krummel-Lorenz, Brigitte, additional, Lauffer, Felix, additional, Launay, David, additional, Li, Mengtao, additional, Litinsky, Ira, additional, Loyo, Esthela, additional, Cerinic, Marco Matucci, additional, Meroni, Pierluigi, additional, Midtvedt, Øyvind, additional, Mihai, Carmen Marina, additional, Montecucco, Carlomaurizio, additional, Montoya, Fabiana, additional, Morgiel, Ewa, additional, Mouthon, Luc, additional, Müller-Ladner, Ulf, additional, Nielsen, Henrik, additional, Opris, Daniela, additional, Ortiz-Santamaria, Vera, additional, Otsa, Kati, additional, Pileckyte, Margarita, additional, Pisarri, Simonetta, additional, Popescu, Monica, additional, Pozzi, Maria Rosa, additional, Puppo, Francesco, additional, Radominski, Sebastião Cezar, additional, Riccieri, Valeria, additional, Rosato, Edoardo, additional, Rozman, Blaz, additional, Rubio, Silvia Rodriguez, additional, Rugiene, Rita, additional, Saar, Petra, additional, Salvador, Maria João, additional, Seidel, Matthias, additional, Sokolik, Renata, additional, Solanki, Kamal, additional, Stamenkovic, Bojana, additional, Stankovic, Aleksandra, additional, Stebbings, Simon, additional, Strauss, Gitte, additional, Sulli, Alberto, additional, Szamosi, Szilvia, additional, Szechinski, Jacek, additional, Szmyrka-Kaczmarek, Magdalena, additional, Szücs, Gabriella, additional, Tanaseanu, Cristina-Mihaela, additional, Tiglea, Isabela, additional, Tyndall, Alan, additional, Ughi, Nicola, additional, Uprus, Maria, additional, Vacca, Alessandra, additional, Varju, Cecilia, additional, Venalis, Algirdas, additional, Venalis, Paulius, additional, Walker, Ulrich A., additional, Widuchowska, Malgorzata, additional, Wiland, Piotr, additional, Wuttge, Dirk M., additional, Zeni, Silvana, additional, and Zenone, Thierry, additional

Published
2015
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21. Abnormalities of Insulin-Like Growth Factor-I Signaling and Impaired Cell Proliferation in Osteoblasts from Subjects with Osteoporosis

Author

Perrini, Sebastio, primary, Natalicchio, Annalisa, additional, Laviola, Luigi, additional, Cignarelli, Angelo, additional, Melchiorre, Mariangela, additional, De Stefano, Francesca, additional, Caccioppoli, Cristina, additional, Leonardini, Anna, additional, Martemucci, Sabina, additional, Belsanti, Gaetana, additional, Miccoli, Sara, additional, Ciampolillo, Anna, additional, Corrado, Ada, additional, Cantatore, Francesco Paolo, additional, Giorgino, Riccardo, additional, and Giorgino, Francesco, additional

Published
2007
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22. Long-Term Follow-up of 320 Chilren Born to Mothers with Systemic Autoimmune Diseases: A Multicentre Survey from 24 Rheumatology Centers in Italy

Author

Tincani, Angela, Romeo, Nicoletta, Sabbadini, Maria Grazia, Baldissera, Elena, Campochiaro, Corrado, Gabrielli, Armando, Zuliani, Lucia, Benigno, Carolina, Foti, Rosario, Visalli, Elisa, Maier, Armin, Vivaldelli, Ester, Afeltra, Antonella, Vadacca, Marta, Malavolta, Nazzarena, Trevisani, Marica, Mosca, Marta, Tani, Chiara, Signorini, Viola, Montecucco, Carlomaurizio, Caporali, Roberto, Véronique Ramoni, Brucato, Antonio, Sebastiani, Gian Domenico, Minisola, Giovanni, Prevete, Imma, Paolazzi, Giuseppe, Peccatori, Susanna, Salvarani, Carlo, Pazzola, Giulia, Govoni, Marcello, Padovan, Melissa, Cutolo, Maurizio, Meroni, Marianna, Selmi, Carlo, Generali, Elena, Ruffatti, Amelia, Doria, Andrea, Larosa, Maddalena, Favaro, Maria, Olivieri, Ignazio, D Angelo, Salvatore, Cantatore, Francesco Paolo, Corrado, Ada, Perricone, Roberto, Conigliaro, Paola, Sinigaglia, Luigi, Meroni, Pier Luigi, Chighizola, Cecilia B., Gerosa, Maria, Gerli, Roberto, Bartoloni-Bocci, Elena, Dall Ara, Francesca, Rodrigues, Marilia, Carini, Chiara, Andreoli, Laura, Nalli, Cecilia, and Lazzaroni, Maria Grazia

23. Gender differences in early systemic sclerosis patients: A report from the EULAR scleroderma trials and research group (EUSTAR) database

Author

Carreira, Patricia E, Carmona, Loreto, Joven, Beatriz E, Loza, Estíbaliz, Andreu, Jose Luis, Riemekasten, Gabriela, Vettori, Serena, Balbir-Gurman, Alexandra, Airò, Paolo, Walker, Ulrich A, Damjanov, Nemanja, Matucci-Cerinic, Marco, Ananieva, Lidia P, Rednic, Simona, Czirják, László, Distler, Oliver, Farge, Dominique, Hesselstrand, Roger, Corrado, Ada, Caramaschi, Paola, Tikly, Mohammed, Allanore, Yannick, Eustar, Coauthors, Sulli, Alberto, Carreira, Pe, Carmona, L, Joven, Be, Loza, E, Andreu, Jl, Riemekasten, G, Vettori, S, Balbir-Gurman, A, Airò, P, Walker, Ua, Damjanov, N, Matucci-Cerinic, M, Ananieva, Lp, Rednic, S, Czirják, L, Distler, O, Farge, D, Hesselstrand, R, Corrado, A, Caramaschi, P, Tikly, M, Allanore, Y, University of Zurich, Carreira, Patricia E, and Chizzolini, Carlo

Subjects
Lung Diseases, Male, 0301 basic medicine, Databases, Factual, 2745 Rheumatology, Acute-Phase Proteins/analysis, 610 Medicine & health, Severity of Illness Index, Scleroderma, Databases, 03 medical and health sciences, Sex Factors, 0302 clinical medicine, Scleroderma, Limited, Risk Factors, Humans, Limited, skin and connective tissue diseases, Lung Diseases/diagnosis/etiology, Factual, Diffuse/blood/complications/diagnosis/immunology, Autoantibodies, ddc:616, 030203 arthritis & rheumatology, 2403 Immunology, integumentary system, Raynaud Disease/diagnosis/etiology, 10051 Rheumatology Clinic and Institute of Physical Medicine, Nuclear Proteins, Raynaud Disease, Health Status Disparities, Acute-Phase Proteins, Biomarkers, Cross-Sectional Studies, Disease Progression, Female, Prognosis, Scleroderma, Diffuse, Diffuse, 030104 developmental biology, DNA Topoisomerases, Type I, 2723 Immunology and Allergy, Nuclear Proteins/immunology, Autoantibodies/blood, Limited/blood/complications/diagnosis/immunology, Biomarkers/blood
Abstract

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (

24. Racial differences in systemic sclerosis disease presentation: A European Scleroderma Trials and Research group study

Author

Jaeger, Veronika K, Tikly, Mohammed, Dong, Xu, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Mengtao, Li, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich, A, Randone, Sb, Bannert, B, Iannone, F, Maurer, B, Jordan, S, Dobrota, R, Becker, M, Mihai, C, Becvarare, R, Tomčík, M, Bielecka, Ok, Gindzienska-Sieskiewicz, E, Karaszewska, K, Cutolo, M, Pizzorni, C, Paolino, S, Sulli, A, Ruaro, B, Alessandri, E, Riccardi, A, Giacco, V, Messitini, V, Irace, R, Kedor, C, Casteleyn, V, Hilger, J, Hoeppner, J, Rednic, S, Szabo, I, Petcu, A, Avouac, J, Camelia, F, Desbas, C, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Cavagna, L, Stork, J, Inanc, M, Joven, Be, Novak, S, Anic, F, Varju, C, Minier, T, Chizzolini, C, Allai, D, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Dolnicar, As, Coleiro, B, Gabrielli, A, Manfredi, L, Benfaremo, D, Ferrarini, A, Bancel, Df, Hij, A, Lansiaux, P, Lazzaroni, Mg, Hesselstrand, R, Wuttge, D, Andréasson, R, Martinovic, D, Bozic, I, Radic, M, Braun-Moscovici, Y, Monaco, Al, Furini, F, Hunzelmann, N, Moinzadeh, P, Pellerito, R, Caimmi, C, Bertoldo, E, Morovic-Vergles, J, Culo, Im, Pecher, Ac, Santamaria, Vo, Heitmann, S, Codagnone, M, Pflugfelder, J, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Hasler, P, Kretschmar, S, Kohm, M, Bajocchi, G, Salvador, Mj, Silva, Japd, Stamenkovic, B, Stankovic, A, Selmi, Cf, Santis, M, Ceribelli, A, Garzanova, L, Koneva, O, Starovoytova, M, Herrick, A, Puppo, F, Negrini, S, Murdaca, G, Engelhart, M, Szücs, G, Szamosi, S, de la Puente, C, Grande, Cs, Villanueva, Mjg, Midtvedt, Sø, Hoffmann-Vold, Am, Launay, D, Sobanski, V, Riccieri, V, Vasile, M, Ionescu, Rm, Opris, D, Sha, A, Woods, A, Gheorghiu, Am, Bojinca, M, Sunderkötter, C, Ehrchen, J, Ingegnoli, F, Mouthon, L, Dunogue, B, Chaigne, B, Legendre, P, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, von Mühlen CA, Pozzi, Mr, Eyerich, K, Lauffer, F, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Vanthuyne, M, Frédéric, H, Alegre-Sancho, Jj, Aringer, M, Herrmann, K, Günther, C, Westhovens, R, Langhe, E, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Üprus, M, Otsa, K, Yavuz, S, Granel, B, Radominski, Sc, De, C, Müller, S, Azevedo, Vf, Mendoza, F, Busquets, J, Popa, S, Agachi, S, Zenone, T, Pileckyte, M, Stebbings, S, Mathieu, A, Vacca, A, Sampaio-Barros, Pd, Stamp, L, Solanki, K, Silva, C, Schollum, J, Barns-Graham, H, Veale, D, O'Rourke, M, Loyo, E, Tineo, C, Paulino, G, Mohamed, Waaa, Rosato, E, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Visalli, E, Benenati, A, Amato, G, Ancuta, C, Villiger, P, Adler, S, Fröhlich, J, Kayser, C, Eduardo, Al, Fathi, N, Alii, S, Ahmed, M, Hasaneen, S, Hakeem, Ee, de la PG, Lefebvre, P, Martin, Jjg, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Galdo, Fd, Abignano, G, Eng, S, Seskute, G, Butrimiene, I, Rugiene, R, Karpec, D, Pascal, M, Kerzberg, E, Bianchi, W, Bianchi, Bv, Bianchi, Dv, Barcellos, Y, Castellví, I, Millan, M, Limonta, M, Rimar, D, Rosner, I, Slobodin, G, Couto, M, Spertini, F, Ribi, C, Buss, G, Marcoccia, A, Bondanini, F, Ciani, A, Kahl, S, Hsu, Vm, Martin, T, Poindron, V, Meghit, K, Moiseev, S, Novikov, P, Chung, L, Kolstad, K, Stark, M, Schmeiser, T, Thiele, A, Majewski, D, Zdrojewski, Z, Zaneta, S, Wierzba, K, Martínez-Barrio, J, López-Longo, Fj, Bernardino, V, Moraes-Fontes, Mf, Rodrigues, Ac, Riemekasten, G, Sommerlatte, S, Jendreck, S, Arnold, S, Levy, Y, Rezus, E, Cardoneanu, A, Burlui, Am, Pamuk, On, Puttini, Ps, Talotta, R, Bongiovanni, S, Poormoghim, H, Andalib, E, Almasi, S, Kötter, I, Krusche, M, Cuomo, G, Danzo, F, Masini, F, Gaches, F, Michaud, M, Cartos, F, Belloli, L, Casu, C, Sfikakis, P, Tektonidou, M, Furst, D, Feldman, Gr, Ramazan, Am, Nurmambet, E, Miroto, A, Suta, C, Andronache, I, Huizinga, Twj, de Vries-Bouwstra, J., Chizzolini, Carlo, Jaeger, Veronika K, Tikly, Mohammed, Xu, Dong, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Li, Mengtao, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich A, University of Zurich, Cerinic, Marco Matucci, Walker Ulrich, A, Randone, Silvia Bellando, Bannert, Bettina, Iannone, Florenzoaa, Maurer, Brittaab, Jordan, Suzanaab, Dobrota, Rucsandraab, Becker, Mikeab, Mihai, Carinaa, Becvarare, Radima, Tomcik, Michala, Bielecka, Otylia Kowala, Gindzienska-Sieskiewicz, Ewaa, Karaszewska, Katarzynaa, Cutolo, Maurizioa, Pizzorni, Carmena, Paolino, Sabrinaae, Sulli, Albertoa, Ruaro, Barbara, Alessandri, Elisa, Riccardi, Antonella, Giacco, Veronica, Messitini, Valentina, Irace, Rosaria, Kedor, Claudia, Casteleyn, Vincent, Hilger, Julia, Hoeppner, Jakob, Rednic, Simona, Szabo, Iulia, Petcu, Ana, Avouac, Jérome, Camelia, Frantz, Desbas, Carole, Vlachoyiannopoulos, Panayioti, Montecucco, Carlo Maurizio, Caporali, Roberto, Cavagna, Lorenzo, Stork, Jiri, Inanc, Murat, Joven, Beatriz E., Novak, Srdan, Anic, Felina, Varju, Cecilia, Minier, Tunde, Allai, Daniela, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Dolnicar, Alenka Sipek, Coleiro, Bernard, Gabrielli, Armando, Manfredi, Lucia, Benfaremo, Devi, Ferrarini, Alessia, Bancel, Dominique Farge, Hij, Adrian, Lazzaroni, Maria Grazia, Hesselstrand, Roger, Wuttge, Dirk, Andréasson, Kristofer, Martinovic, Duska, Bozic, Ivona, Radic, Mislav, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Furini, Federica, Hunzelmann, Nicola, Moinzadeh, Pia, Pellerito, Raffaele, Caimmi, Cristian, Bertoldo, Eugenia, Morovic-Vergles, Jadranka, Culo, Ivana Melanie, Pecher, Ann-Christian, Santamaria, Vera Ortiz, Heitmann, Stefan, Codagnone, Medeleine, Pflugfelder, Johanne, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthia, Hasler, Paul, Kretschmar, Samuel, Kohm, Michaela, Bajocchi, Gianluigi, Salvador, Maria João, Da Silva, JoséAntonio Pereira, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Ceribelli, Angela, Garzanova, Ludmila, Koneva, Olga, Starovoytova, Maya, Herrick, Ariane, Puppo, Francesco, Negrini, Simone, Murdaca, Giuseppe, Engelhart, Merete, Szücs, Gabriela, Szamosi, Szilvia, De La Puente, Carlo, Grande, Cristina Sobrino, Villanueva, Maria Jesus Garcia, Midtve, Øyvindbw, Hoffmann-Vold, Anna-Mariabw, Launay, Davidbx, Sobanski, Vincentbx, Riccieri, Valeriaby, Vasile, Massimilianoby, Stefantoni, Katia, Ionescu, Ruxandra Maria, Opris, Daniela, Sha, Ami, Woods, Adrianne, Gheorghiu, Ana Maria, Bojinca, Mihai, Sunderkötter, Cord, Ehrchen, Jan, Ingegnoli, Francesca, Mouthon, Luc, Dunogue, Bertrand, Chaigne, Benjamin, Legendre, Paul, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Von Mühlen, Carlos Alberto, Pozzi, Maria Rosa, Eyerich, Kilian, Lauffer, Felix, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Vanthuyne, Marie, Frédéric, Houssiau, Alegre-Sancho, Juan Jose, Aringer, Martin, Herrmann, Kristine, Günther, Claudia, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastião Cezar, De Souza Müller, Carolina, Feijóazevedo, Valderílio, Mendoza, Fabian, Busquets, Joanna, Popa, Sergei, Agachi, Svetlana, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Jordan, Sarah, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Silva, Cherumi, Schollum, Joanne, Barns-Graham, Helen, Veale, Dougla, O'Rourke, Marie, Loyo, Esthela, Tineo, Carmen, Paulino, Glenny, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Fröhlich, Johanne, Kayser, Cristiane, Eduardo, Andrade Lui, Fathi, Nihal, Alii, Safa, Ahmed, Marrow, Hasaneen, Samar, El Hakeem, Eman, De La Peña Lefebvre, Paloma García, Martin, Jorge Juan Gonzalez, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Del Galdo, Francesco, Abignano, Giuseppina, Eng, Sookho, Seskute, Goda, Butrimiene, Irena, Rugiene, Rita, Karpec, Diana, Pascal, Melanie, Kerzberg, Eduardo, Bianchi, Washington, Bianchi, Breno Valdetaro, Bianchi, Dante Valdetaro, Barcellos, Yeda, Castellví, Ivan, Millan, Milena, Limonta, Massimiliano, Rimar, Doron, Rosner, Itzhak, Slobodin, Gleb, Couto, Maura, Spertini, Françoi, Ribi, Camillo, Buss, Guillaume, Marcoccia, Antonella, Bondanini, Francesco, Ciani, Aldo, Kahl, Sarah, Hsu, Vivien M., Martin, Thierry, Poindron, Vincent, Meghit, Kilifa, Moiseev, Sergey, Novikov, Pavel, Chung, Lori, Kolstad, Kathleen, Stark, Marianna, Schmeiser, Tim, Thiele, Astrid, Majewski, Dominik, Zdrojewski, Zbigniew, Zaneta, Smolenska, Wierzba, Karol, Martínez-Barrio, Julia, López-Longo, Francisco Javier, Bernardino, Vera, Moraes-Fontes, Maria Francisca, Rodrigues, Ana Catarina, Riemekasten, Gabriela, Sommerlatte, Sabine, Jendreck, Sebastian, Arnold, Sabrina, Levy, Yair, Rezus, Elena, Cardoneanu, Anca, Burlui, Alexandra Maria, Pamuk, Omer Nuri, Puttini, Piercarlo Sarzi, Talotta, Rossella, Bongiovanni, Sara, Poormoghim, Hadi, Andalib, Elham, Almasi, Simin, Kötter, Ina, Krusche, Matrin, Cuomo, Giovanna, Danzo, Fiammetta, Masini, Francesco, Gaches, Franci, Michaud, Martin, Cartos, Florian, Belloli, Laura, Casu, Cinzia, Sfikakis, Petro, Tektonidou, Maria, Furst, Daniel, Feldman, Gary R., Ramazan, Ana-Maria, Nurmambet, Emel, Miroto, Amalia, Suta, Cristina, Andronache, Iulia, Huizinga, Tom W. J., De Vries-Bouwstra, Jeska, and Walker, Ulrich A.

Subjects
Male, Vital capacity, Organ manifestations, systemic sclerosis, Type I, race difference, Systemic scleroderma, Gastroenterology, Scleroderma, immunology, 0302 clinical medicine, Diffusing capacity, middle aged, pulmonary hypertension, Medicine, Pharmacology (medical), 030212 general & internal medicine, organ manifestations, races, skin and connective tissue diseases, Lung, race, pathophysiology, African Continental Ancestry Group, ddc:616, integumentary system, disease course, Hazard ratio, Races, 10051 Rheumatology Clinic and Institute of Physical Medicine, Pulmonary, Middle Aged, Blacks, cohort analysis, Autoantibodie, 3. Good health, Asians, female, priority journal, DNA Topoisomerases, Type I, Black, centromere, Cohort, Hypertension, organ manifestation, Systemic sclerosis, Female, systemic sclerosi, Human, Adult, Asian Continental Ancestry Group, medicine.medical_specialty, Hypertension, Pulmonary, European Continental Ancestry Group, Black People, 610 Medicine & health, complication, Caucasian, White People, Article, lung, 03 medical and health sciences, Black person, Rheumatology, Asian People, forced vital capacity, Internal medicine, geographic distribution, Humans, controlled study, human, DNA topoisomerase, Aged, Autoantibodies, 030203 arthritis & rheumatology, Scleroderma, Systemic, Asian, business.industry, Whites, Systemic, Odds ratio, medicine.disease, Pulmonary hypertension, major clinical study, mortality, clinical feature, business, DNA Topoisomerases, autoantibody
Abstract

Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.

Published
2020

25. The incidence of cardiovascular events in Italian patients with systemic lupus erythematosus is lower than in North European and American cohorts: Implication of disease-associated and traditional risk factors as emerged by a 16-year retrospective GIRRCS study

Author

Domenico Paolo Emanuele Margiotta, L. Pierro, Antonella Riccardi, Roberto Giacomelli, Serena Fasano, Gabriele Valentini, Pier Luigi Meroni, Antonella Afeltra, Francesco Paolo Cantatore, Roberta Gualtierotti, Onorina Berardicurti, Daniela Iacono, Ada Corrado, Fasano, Serena, Margiotta, Domenico Paolo, Gualtierotti, Roberta, Corrado, Ada, Berardicurti, Onorina, Iacono, Daniela, Pierro, Luciana, Riccardi, Antonella, Giacomelli, Roberto, Cantatore, Francesco Paolo, Meroni, Pier Luigi, Afeltra, Antonella, and Valentini, Gabriele

Subjects
Adult, Male, Cardiovascular events, Incidence, Systemic lupus erythematosus, Environment, Female, Humans, Italy, Middle Aged, Patient Care Management, Retrospective Studies, Risk Factors, Cardiovascular Diseases, Lupus Erythematosus, Systemic, Medicine (all), medicine.medical_specialty, Observational Study, Disease, Cardiovascular event, Systemic lupus erythematosu, 030204 cardiovascular system & hematology, Observational period, 03 medical and health sciences, cardiovascular events, 0302 clinical medicine, systemic lupus erythematosus, Retrospective Studie, Internal medicine, Cardiovascular Disease, medicine, Cumulative incidence, 030203 arthritis & rheumatology, First episode, Lupus Erythematosus, business.industry, Incidence (epidemiology), Risk Factor, Systemic, Retrospective cohort study, General Medicine, medicine.disease, Cohort, business, Research Article, Human
Abstract

Previous study from our group has pointed out a lower number of cardiovascular (CV) events in Italian patients with systemic lupus erythematosus (SLE) than in North European and American ones. This study aims to assess the incidence of the first CV event in a large, multicenter, Italian cohort of patients with SLE and search for differences in disease and traditional risk factors among distinct cohorts. Clinical charts of SLE patients consecutively admitted to 5 Italian rheumatologic centers from November 1st 2000 to December 31st 2015 and free of CV events at baseline were retrospectively studied. CV cumulative incidence (ie, the proportion of patients who experienced a new CV event over the follow-up period) and CV incidence rate (ie, the number of events in the cohort divided by the total number of years at risk) were evaluated. The detected incidences were compared with those reported in SLE cohorts from other countries. The median duration of follow-up was 6 years (IQR=3-11). During the observational period, 37 (cumulative incidence=7.2%) patients had a first episode of CV event with an incidence rate of 10.1/1000 person-years. The CV cumulative incidence and incidence rate detected in our Italian cohort were lower than those from most North European and American cohorts, characterized by a high impact of traditional risk factors. Nevertheless, the cumulative incidence was similar to that reported in a Spanish cohort with a high frequency of traditional risk factors (geographic impact), while the incidence rate was only slightly higher than that in the Baltimore cohort, which is characterized by a strict follow-up of patients (medical impact). Our results confirmed that Italian lupus patients have a low incidence of CV events. Moreover, the geographic origin, traditional risk factors, and medical approach appear to have an impact on CV disease in SLE. Abbreviations: ACR = American College of Rheumatology, aPL = antiphospholipid antibody, ASA = aspirin, CV = cardiovascular, HCQ = hydroxychloroquine, SCORE = Systematic COronary Risk Evaluation, SLE = systemic lupus erythematosus.

Published
2018

26. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

Author

Maurer, B., Graf, N., Michel, B. A., Muller Ladner, U., Czirjak, L., Denton, C. P., Tyndall, A., Metzig, C., Lanius, V., Khanna, D., Distler, O., Arner, I. H., Cerinic, M. M., Guiducci, S., Walker, U., Lapadula, G., Iannone, F., Becvar, R., Sierakowsky, S., Bielecka, O. K., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Vettori, S., Riemekasten, G., Rednic, S., Nicoara, I., Kahan, A., Allanore, Y., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Carreira, P. E., Novak, S., Varju, C., Chizzolini, C., Kucharz, E. J., Kotulska, A., Kopec Medrek, M., Widuchowska, M., Cozzi, F., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Hij, A., Airo, P., Hesselstrand, R., Scheja, A., Wollheim, F., Martinovic, D., Gurman, A. B., Braun Moscovici, Y., Govoni, Marcello, LO MONACO, Andrea, Hunzelmann, N., Pellerito, R., Bambara, L. M., Caramaschi, P., Black, C., Damjanov, N., Santamaria, V. O., Heitmann, S., Krasowska, D., Seidel, M., Oleszowsky, M., Burkhardt, H., Himsel, A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Starovoytova, M. N., Ananieva, L. P., Scorza, R., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szucs, G., Mendoza, A. Z., Buijdos, C. d. l. P., Sifuentes Giraldo, W. A., Midtvedt, O., Garen, T., Hachulla, E., Launay, D., Valesini, G., Riccieri, V., Ionescu, R. M., Opris, D., Groseanu, L., Wigley, F. M., Mihai, C. M., Cornateanu, R. S., Ionitescu, R., Gherghe, A. M., Gorga, M., Dobrota, R., Bojinca, M., Schett, G., Distler, J. H., Meroni, P., Zeni, P., Mouthon, L., Keyser, F. D., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Szechinski, J., Wiland, P., Szmyrka Kaczmarek, M., Sokolik, R., Morgiel, E., Krummel Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anic, B., Baresic, M., Mayer, M., Radominski, S. C., Muller, C. d. S., Azevedo, V. F., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Zenone, T., Highton, J., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Pisarri, S., Tanaseanu, C. M., Popescu, M., Dumitrascu, A., Tiglea, I., Chirieac, R., Ancuta, C., Furst, D. E., Kafaja, S., Lefebvre, P. G. d. l. P., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Kerzberg, E., Montoya, F., Cosentino, V., Chizzolini, Carlo, Maurer, Britta, Graf, Nicole, Michel, Beat A, Müller Ladner, Ulf, Czirják, László, Denton, Christopher P, Tyndall, Alan, Metzig, Carola, Lanius, Vivian, Khanna, Dinesh, Distler, Oliver, Tarner, Ingo H, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriele, Rednic, Simona, Nicoara, Ileana, Kahan, André, Allanore, Yannick, Vlachoyiannopoulos, P, Montecucco, Carlomaurizio, Caporali, Roberto, Carreira, Patricia E, Novak, Srdan, Varju, Cecilia, Kucharz, Eugene J, Kotulska, Anna, Kopec Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Gurman, Alexandra Balbir, Braun Moscovici, Yolanda, Govoni, M, Monaco, Andrea Lo, Hunzelmann, Nicola, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthia, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J, Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N, Ananieva, Lidia P, Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Mendoza, Antonio Zea, Buijdos, Carlos de la Puente, Giraldo, Walter A. Sifuente, Midtvedt, Øyvind, Garen, Torhild, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Mihai, Carmen M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Cantatore, Francesco P, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R, Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C, Müller, Carolina de Souza, Azevedo, Valderílio F, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Dougla, O’Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina Mihaela, Popescu, Monica, and University of Zurich

Subjects
Genetics and Molecular Biology (all), Male, Time Factors, Databases, Factual, systemic sclerosis, 2745 Rheumatology, computer.software_genre, Biochemistry, Severity of Illness Index, Outcomes Research, Qualitative Research, Systemic Sclerosis, Adult, Cohort Studies, Creatine Kinase, Decision Support Techniques, Deglutition Disorders, Dyspnea, Female, Fibrosis, Humans, Logistic Models, Middle Aged, Multivariate Analysis, Scleroderma, Diffuse, Sex Factors, Skin, Synovitis, Disease Progression, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Immunology and Allergy, Medicine (all), Scleroderma, skin fibrosis, skin and connective tissue diseases, ddc:616, EUSTAR, Univariate analysis, Database, integumentary system, 10051 Rheumatology Clinic and Institute of Physical Medicine, Orvostudományok, Diffuse, Connective tissue disease, Cohort, 2723 Immunology and Allergy, Cohort study, medicine.medical_specialty, 610 Medicine & health, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, NO, outcomes research, qualitative research, Databases, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, medicine, Factual, 2403 Immunology, business.industry, medicine.disease, business, computer
Abstract

ObjectivesTo identify predictive parameters for the progression of skin fibrosis within 1 year in patients with diffuse cutaneous SSc (dcSSc).MethodsAn observational study using the EUSTAR database was performed. Inclusion criteria were dcSSc, American College of Rheumatology (ACR) criteria fulfilled, modified Rodnan skin score (MRSS) ≥7 at baseline visit, valid data for MRSS at 2nd visit, and available follow-up of 12±2 months. Worsening of skin fibrosis was defined as increase in MRSS >5 points and ≥25% from baseline to 2nd visit. In the univariate analysis, patients with progressive fibrosis were compared with non-progressors, and predictive markers with pResultsA total of 637 dcSSc patients were eligible. Univariate analyses identified joint synovitis, short disease duration (≤15 months), short disease duration in females/patients without creatine kinase (CK) elevation, low baseline MRSS (≤22/51), and absence of oesophageal symptoms as potential predictors for progressive skin fibrosis. In the multivariate analysis, by employing combinations of the predictors, 17 models with varying prediction success were generated, allowing cohort enrichment from 9.7% progressive patients in the whole cohort to 44.4% in the optimised enrichment cohort. Using a second validation cohort of 188 dcSSc patients, short disease duration, low baseline MRSS and joint synovitis were confirmed as independent predictors of progressive skin fibrosis within 1 year resulting in a 4.5-fold increased prediction success rate.ConclusionsOur study provides novel, evidence-based criteria for the enrichment of dcSSc cohorts with patients who experience worsening of skin fibrosis which allows improved clinical trial design.

Published
2014

27. Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis

Author

Patricia E Carreira, Carmona, L., Joven, B. E., Loza, E., Andreu, J. L., Riemekasten, G., Vettori, S., Allanore, Y., Balbir-Gurman, A., Airò, P., Walker, U. A., Damjanov, N., Ananieva, L. P., Rednic, S., Czirják, L., Distler, O., Farge, D., Hesselstrand, R., Corrado, A., Caramaschi, P., Tikly, M., Matucci-Cerinic, M., Carreira, Patricia E., Carmona, Loreto, Joven, Beatriz E., Loza, Estibaliz, Andreu, Josã© Lui, Riemekasten, Gabriela, Vettori, Serena, Allanore, Yannick, Balbir gurman, Alexandra, Airã², Paolo, Walker, Ulrich A., Damjanov, Nemanja, Ananieva, Lidia P., Rednic, Simona, Czirjã¡k, Lã¡szlã³, Distler, Oliver, Farge, Dominique, Hesselstrand, Roger, Corrado, Ada, Caramaschi, Paola, Tikly, Mohammed, and Matucci cerinic, Marco

Subjects
Adult, Male, Scleroderma, Systemic, Systole, Immunology, Middle Aged, Pulmonary Artery, Pulmonary arterial hypertension, Echocardiography, Doppler, Systemic sclerosi, Cross-Sectional Studies, Logistic Models, Rheumatology, Right heart catheterisation, Echocardiography, Immunology and Allergy, Humans, Female, Aged
Abstract

Objective. To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Dopplerechocardiography (TTE) in patients with early systemic sclerosis (SSc). Methods. A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with < 3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected. Elevated sPAP was defined as sPAP > 40 mmHg on baseline TTE. First visit SSc related variables, including disease subsets, antibodies and visceral involvement, were examined. Results. From 1,188 patients, 81% were women. Mean (SD) age at first non-RP symptom was 50 (14) years, 55% had limited cutaneous SSc (lcSSc) and 42% active disease. Elevated sPAP was found in 17% of patients, both lcSSc and diffuse cutaneous SSc (dc- SSc). In lcSSc, older age at first non- RP symptom, ACA positivity, joint contractures, restrictive defect and lower DLCO, were independently associated with elevated sPAP. In dcSSc, older age at first non-RP symptom, longer time between RP onset and first non-RP symptom, digital ulcers, cardiac blocks, and proteinuria were associated with elevated sPAP. Conclusion. The prevalence of elevated sPAP on TTE in early SSc patients is considerable. Association with cardiac, lung and renal involvement suggests that, although some patients might have pulmonary arterial hypertension, others may present pulmonary hypertension secondary to lung or heart involvement. Our findings emphasise the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.

28. Clinical and subclinical atherosclerosis in patients with systemic sclerosis: an observational, multicentre study of GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale).

Author

Liakouli V, Verde I, Ruscitti P, Di Vico C, Ruggiero A, Mauro D, Forte G, Navarini L, Di Donato S, Bearzi P, Minerba M, Bertolini N, Favoino E, Destro Castaniti GM, D'Alessandro R, Berlengiero V, Italiano N, Bellisai F, Caso F, Guggino G, Corrado A, Triggianese P, Lo Gullo A, Mandraffino G, Cantarini L, Cipriani P, Cantatore FP, Chimenti MS, Perosa F, Iagnocco A, Docimo L, Giacomelli R, and Ciccia F

Subjects
Humans, Female, Male, Middle Aged, Italy epidemiology, Aged, Adult, Prevalence, Risk Factors, Aged, 80 and over, Young Adult, Ultrasonography, Doppler, Asymptomatic Diseases, Carotid Artery Diseases epidemiology, Carotid Artery Diseases diagnostic imaging, Plaque, Atherosclerotic diagnostic imaging, Plaque, Atherosclerotic epidemiology, Scleroderma, Systemic epidemiology, Scleroderma, Systemic complications, Atherosclerosis epidemiology, Atherosclerosis diagnostic imaging, Atherosclerosis etiology, Carotid Intima-Media Thickness
Abstract

Objectives: Conflicting results about clinical and subclinical atherosclerosis in systemic sclerosis (SSc) and the associated risk factors have been reported. Hence, we aimed to determine the prevalence of clinical and subclinical atherosclerosis in a large number of Italian SSc patients and the associated risk factors., Methods: This study included 613 SSc patients from 11 Italian tertiary Rheumatologic Units. All patients underwent full history taking, clinical examination, and relevant laboratory and radiological investigations. Doppler ultrasonography (US) of the common carotid and upper and lower limbs was performed to measure carotid and femoral intima-media thickness (cIMT and fIMT), and carotid and peripheral atheroma plaques. Doppler US of the brachial artery was performed to measure flow-mediated dilatation (FMD)., Results: Patients were mostly women (91.4%) with a median age of 61 years (range, 20-100); a median disease duration of 14 years (range, 0-77) from the onset of the first non-Raynaud's phenomenon (RP); 9.3% had a history of clinical atherosclerosis (9 stable/unstable angina, 21 myocardial infarctions, 24 heart failure, 3 strokes, 8 transient ischaemic attack, 6 intermittent claudication, 10 atrial thrombo-embolism). In 37.1% of patients, subclinical atherosclerosis was detected, after excluding those with a history of clinical atherosclerosis. The prevalence of clinical and subclinical atherosclerosis was higher than that reported by the European Society of Cardiology and observational studies that enrolled Italian healthy individuals as a control group, respectively., Conclusions: A higher prevalence of clinical and subclinical atherosclerosis was detected in SSc Italian patients and correlated with traditional and SSc-related risk factors.

Published
2024
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29. Gender differences in early systemic sclerosis patients: a report from the EULAR scleroderma trials and research group (EUSTAR) database.

Author

Carreira PE, Carmona L, Joven BE, Loza E, Andreu JL, Riemekasten G, Vettori S, Balbir-Gurman A, Airò P, Walker UA, Damjanov N, Matucci-Cerinic M, Ananieva LP, Rednic S, Czirják L, Distler O, Farge D, Hesselstrand R, Corrado A, Caramaschi P, Tikly M, and Allanore Y

Subjects
Acute-Phase Proteins analysis, Autoantibodies blood, Biomarkers blood, Cross-Sectional Studies, DNA Topoisomerases, Type I, Databases, Factual, Disease Progression, Female, Humans, Lung Diseases diagnosis, Lung Diseases etiology, Male, Nuclear Proteins immunology, Prognosis, Raynaud Disease diagnosis, Raynaud Disease etiology, Risk Factors, Scleroderma, Diffuse blood, Scleroderma, Diffuse complications, Scleroderma, Diffuse immunology, Scleroderma, Limited blood, Scleroderma, Limited complications, Scleroderma, Limited immunology, Severity of Illness Index, Sex Factors, Health Status Disparities, Scleroderma, Diffuse diagnosis, Scleroderma, Limited diagnosis
Abstract

Objectives: To describe differences in clinical presentation between men and women in a large group of patients with early (<3 years' duration) systemic sclerosis (SSc) according to disease subsets., Methods: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research database (EUSTAR) was performed. Patients fulfilling preliminary ACR 1980 classification criteria for SSc, with less than 3 years from the first non-Raynaud's symptom at first entry, were selected. A group of patients with less than 3 years from the first SSc symptom, including Raynaud's phenomenon, was also analysed. SSc related variables, including antibodies, SSc subsets, disease activity and organ involvement were included. Descriptive and bivariate analyses were performed., Results: A total of 1,027 patients were included, 90% Caucasian, 80% women, and 40% with diffuse cutaneous disease. In early stages of SSc, men showed more frequently than women active disease, diffuse cutaneous subset, anti-Scl-70 antibodies, elevated acute phase reactants, muscular and pulmonary involvement. Differences between men and women were confirmed in the limited, but not in the diffuse SSc subset. The results were similar when 650 patients with less than three years from the first SSc symptom, including Raynaud's phenomenon, were analysed., Conclusions: In early stages of SSc, men present signs and symptoms of more severe disease. In the limited disease subset, men might appear with clinical features and organ involvement similar to those of the diffuse subgroup. In clinical practice, the identification of such differences might help to select the appropriate management for each particular patient.

Published
2018

30. Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis.

Author

Carreira PE, Carmona L, Joven BE, Loza E, Andreu JL, Riemekasten G, Vettori S, Allanore Y, Balbir-Gurman A, Airò P, Walker UA, Damjanov N, Ananieva LP, Rednic S, Czirják L, Distler O, Farge D, Hesselstrand R, Corrado A, Caramaschi P, Tikly M, and Matucci-Cerinic M

Subjects
Adult, Aged, Cross-Sectional Studies, Female, Humans, Logistic Models, Male, Middle Aged, Scleroderma, Systemic diagnostic imaging, Echocardiography methods, Echocardiography, Doppler methods, Pulmonary Artery physiopathology, Scleroderma, Systemic physiopathology, Systole physiology
Abstract

Objectives: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc)., Methods: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected. Elevated sPAP was defined as sPAP>40 mmHg on baseline TTE. First visit SSc related variables, including disease subsets, antibodies and visceral involvement, were examined., Results: From 1,188 patients, 81% were women. Mean (SD) age at first non-RP symptom was 50 (14) years, 55% had limited cutaneous SSc (lcSSc) and 42% active disease. Elevated sPAP was found in 17% of patients, both lcSSc and diffuse cutaneous SSc (dcSSc). In lcSSc, older age at first non-RP symptom, ACA positivity, joint contractures, restrictive defect and lower DLCO, were independently associated with elevated sPAP. In dcSSc, older age at first non-RP symptom, longer time between RP onset and first non-RP symptom, digital ulcers, cardiac blocks, and proteinuria were associated with elevated sPAP., Conclusions: The prevalence of elevated sPAP on TTE in early SSc patients is considerable. Association with cardiac, lung and renal involvement suggests that, although some patients might have pulmonary arterial hypertension, others may present pulmonary hypertension secondary to lung or heart involvement. Our findings emphasize the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH.

Published
2017

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